Sunday, 17 July 2016

UNDERSTANDING SCELERODEMA


Having spent two weeks at the dermatology department, I observed many skin related diseases, but three appeared on most patients we examined and the most common symptom seen during physical examination was a reddish itchy rash. Skin conditions such as scleroderma, psoriasis and lichen planus were the most common and by statistics I can say that 1 in 4 patients we examined had one of the three diseases. Therefore, it is the purpose of this article to outline scleriderna its definition, etiology, pathogenesis, symptoms, diagnosis and treatment. This article will also highlight complications in relation to the disease, not forgetting differential diagnose.

The terminology ‘scleroderma’ is derived from the Greek words ‘skleros’ hard and ‘derma’ for skin and was first described as a condition of thickened skin by Hippocrates and Carlo Curzio fully described the details of the disease in 1752 on a patient.  It is important to understand that Hippocrates described the condition of thickened skin, but did not call it ‘scleroderma’. Therefore, it was Giovambattisa Fantonetti who first used the term ’skleroderma’ after observing a patient with a ‘dark-leather like skin’ that somehow caused the skin to tighten in 1836 and later on in 1945, Robert H. Goetz coined the systemic manifestation of the disease which he profoundly described as progressive.
Therefore, scleroderma can be defined as a progressive autoimmune characterized by skin hardening. And in line with its history, scleroderma’s cause is unknown, that’s why it is termed as autoimmune because it is suspected that it occurs when ones immune system attacks and destroys  healthy body tissue by mistake and this dramatically changes the function of the immune system which is to protect from disease and infection. And it is a known fact that the cause of scleroderma is as a result of overproduction and accumulation of collagen (main component of connective tissue), but yet what prompts the abnormal collagen production is stil unknown.

And in accordance to its pathogenesis, genetics and environmental factors have been implicated to be in the etiology of scleroderma. While changes in metabolism, immune and microcirculatory disturbance have been associated to the disease, not forgetting abortion, retroviruses, coal dust and chemotherapy.

Furthermore, it is important to understand that scleroderma is divided into; localized (limited) and systemic. Localized scleroderma has a benign course and mainly affects fingers, hands and face while systemic scleroderma is characterized by affecting connective tissues, lesions of the skin, musculoskeletal system and internal organs such as the heart, kidneys and digestive tract.
Furthermore, localized scleroderma can be divided into linear and morphea while systemic also has limited and diffused scleroderma and are characterized by symptoms such as sores on fingertips, weight loss, diarrhea, heartburn, muscle weakness, calcinosis, Raynaud phenomenon, telangiectasia, esophageal dysfunction and sclerodactyly. The above symptoms are important in the diagnosis of localized and systemic scleroderma respectively.

Even though, scleroderma affects many different parts of the body and can appear in many forms, its diagnose is somehow difficult. Despite that being the case, a proper physical examination and blood tests are one of the vital stages in checking if certain antibodies produced in the immune system have elevated the blood levels. Also pulmonary function test, ct scan of scan and echocardiogram of heart are helpful techniques to diagnosis systemic scleroderma due to its ability to cause complication in digestive tract and heart.


In conclusion, no drug has been created to stop the overproduction of collagen, but treatment is directed towards individual symptoms affecting different areas of the body.


extracted from my 2015 fifth student conference Tambov State University